Intracranial extramedullary hematopoiesis in a patient with beta-thalassemia presenting with head trauma

Abstract

Background: Beta-thalassemia is an inherited hemoglobinopathy, whereby reduced or absent expression of beta-globin genes causes impaired erythropoiesis. Extramedullary hematopoiesis (EMH) occurs in 1% of all patients with beta-thalassemia major receiving regular transfusions and is exceedingly rare intracranially. Case Description: We report a case of a male in his 20s with beta thalassemia who presented with head trauma found to have intracranial EMH mimicking multiple extra-axial hematomas. Making the correct diagnosis was critical in avoiding prolonged neuromonitoring and unnecessary interventions. Conclusion: Intracranial extramedullary hematopoietic pseudotumor is an exceedingly rare entity and seldom appears in a neurosurgeon’s differential diagnosis. This case illustrates how this condition can easily mimic an acute intracranial hemorrhage in a patient with beta-thalassemia who presents with head trauma. We review the topic to further inform clinicians who may encounter this condition in their practice.